Inflammatory Myofibroblastic Tumors in Childhood
Inflammatory Myofibroblastic Tumors in Childhood
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DOI：
10.3109/08880018.2013.816810
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发表时间：
2013-10-01
影响因子：
1.7
通讯作者：
Venkatramani, Rajkumar
中科院分区：
文献类型：
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作者：
Mehta, Bhakti;Mascarenhas, Leo;Venkatramani, Rajkumar
炎性肌纤维母细胞瘤（IMT）是一种罕见的中等恶性肿瘤，可发生在身体的任何地方。手术切除是主要治疗手段。我们报告了9名儿童诊断为IMT在我们的机构超过10年的时间。临床症状反映了肿瘤的位置。完全手术切除是治愈的。局部复发发生在手术切缘受累的情况下。1例转移性疾病患者仅接受化疗后获得长期缓解。1例患者发生严重炎症反应并死亡。3年无事件生存率和总生存率（OS）分别为58 +/- 20%和89 +/- 10%。
Inflammatory myofibroblastic tumor (IMT) is a rare tumor of intermediate malignant potential that can occur anywhere in the body. Surgical resection is the principal treatment. We report on nine children diagnosed with IMT at our institution over a 10-year period. Presenting symptoms were reflective of tumor location. Complete surgical resection was curative. Local recurrence occurred in the presence of involved surgical margins. One patient with metastatic disease achieved long-term remission with chemotherapy alone. Severe inflammatory response and death occurred in one patient. The 3-year event free and overall survivals (OS) were 58 +/- 20% and 89 +/- 10% respectively.